University of Illinois at Chicago Chicago, IL, United States
James Love, MD, Elie Ghoulam, MD, MS, Sarang Thaker, MD, Robert Carroll, MD University of Illinois at Chicago, Chicago, IL
Introduction: Primary T-cell Non-Hodgkin lymphoma (NHL) of the gastrointestinal tract (GIT) is a rare, poorly-characterized clinical entity that usually presents with non-specific abdominal complaints. A well-known complication of intestinal NHL is perforation due to chemotherapy, but perforation as a presenting sign of GIT lymphoma is extremely rare. Here we present a case of spontaneous intestinal perforation secondary to primary intestinal T-cell lymphoma.
Case Description/Methods: A 56-year-old man presented with acute severe abdominal pain and nausea. On arrival he was hypotensive and tachycardic. His abdomen was rigid and diffusely tender. Labs were notable for leukocytosis, lactic acidosis, and venous pH of 7.2. Abdominal x-ray and computed tomography (CT) abdomen demonstrated moderate pneumoperitoneum and high-grade small bowel obstruction without identifiable transition point, and he was taken for emergent exploratory laparotomy. Intraoperatively a 3mm perforation within an inflamed segment of the proximal ileum was found. Biopsy of the perforation showed mucosal denudation and gland dropout, ulceration, and marked acute and chronic inflammation. Peritoneal culture was positive for E. coli. Patient underwent diverting loop ileostomy proximal to the site of inflamed ileum and perforation and was admitted to the intensive care unit (ICU) for post-operative management. Post-operative course was complicated by severe protein calorie malnutrition requiring TPN and high ileostomy output. Ileoscopy prior to discharge revealed scalloped mucosa and blunted villi up to 30cm proximal to the stoma and a single hemi-circumferential 5mm ulcer. Biopsies showed atypical lymphoid infiltrates, suspicious for involvement by mature intestinal T-cell lymphoproliferative neoplasm. Subsequent T-cell receptor rearrangement studies were positive for a clonal T-cell population, confirming the diagnosis of intestinal T-cell lymphoma. Staging CTs were negative for metastatic disease. Bone marrow biopsy and peripheral smear were also unremarkable. Patient was ultimately referred to hematology for initiation of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy and continues to be closely followed.
Discussion: Spontaneous intestinal perforation is an extremely rare presentation of primary GIT T-cell lymphoma. This case highlights the importance of early recognition of this uncommon cause of perforation as a crucial step to ensure expedited hematology referral and initiation of appropriate treatment.
Figure: Figure 1: CT Abdomen with thin lucency below the right hemidiaphragm (A) and moderate pneumoperitoneum in the right upper quadrant and anterior abdomen (B) suggestive of moderate pneumoperitoneum.
Figure 2: Ileoscopy of ileum notable for scalloped mucosa with blunted villi up to 30 cm proximal to the stoma (A, B) and a single hemi-circumferential 5 mm ulcer without stigmata of recent bleeding (C,D).
James Love indicated no relevant financial relationships.
Elie Ghoulam indicated no relevant financial relationships.
Sarang Thaker indicated no relevant financial relationships.
Robert Carroll indicated no relevant financial relationships.
James Love, MD, Elie Ghoulam, MD, MS, Sarang Thaker, MD, Robert Carroll, MD. P2004 - Primary Gastrointestinal T-cell Lymphoma Presenting as Spontaneous Perforation Peritonitis: A Case Report, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.